PATHFINDER-CHD: prospective registry on adults with congenital heart disease, abnormal ventricular function, and/or heart failure as a foundation for establishing rehabilitative, prehabilitative, preventive, and health-promoting measures: rationale, aims, design and methods.

BACKGROUND: Adults with congenital heart defects (ACHD) globally constitute a notably medically underserved patient population. Despite therapeutic advancements, these individuals often confront substantial physical and psychosocial residua or sequelae, requiring specialized, integrative cardiological care throughout their lifespan. Heart failure (HF) is a critical challenge in this population, markedly impacting morbidity and mortality. AIMS: The primary aim of this study is to establish a comprehensive, prospective registry to enhance understanding and management of HF in ACHD. Named PATHFINDER-CHD, this registry aims to establish foundational data for treatment strategies as well as the development of rehabilitative, prehabilitative, preventive, and health-promoting interventions, ultimately aiming to mitigate the elevated morbidity and mortality rates associated with congenital heart defects (CHD). METHODS: This multicenter survey will be conducted across various German university facilities with expertise in ACHD. Data collection will encompass real-world treatment scenarios and clinical trajectories in ACHD with manifest HF or at risk for its development, including those undergoing medical or interventional cardiac therapies, cardiac surgery, inclusive of pacemaker or ICD implantation, resynchronization therapy, assist devices, and those on solid organ transplantation. DESIGN: The study adopts an observational, exploratory design, prospectively gathering data from participating centers, with a focus on patient management and outcomes. The study is non-confirmatory, aiming to accumulate a broad spectrum of data to inform future hypotheses and studies. PROCESSES: Regular follow-ups will be conducted, systematically collecting data during routine clinical visits or hospital admissions, encompassing alterations in therapy or CHD-related complications, with visit schedules tailored to individual clinical needs. ASSESSMENTS: Baseline assessments and regular follow-ups will entail comprehensive assessments of medical history, ongoing treatments, and outcomes, with a focus on HF symptoms, cardiac function, and overall health status. DISCUSSION OF THE DESIGN: The design of the PATHFINDER-CHD Registry is tailored to capture a wide range of data, prioritizing real-world HF management in ACHD. Its prospective nature facilitates longitudinal data acquisition, pivotal for comprehending for disease progression and treatment impacts. CONCLUSION: The PATHFINDER-CHD Registry is poised to offer valuable insights into HF management in ACHD, bridging current knowledge gaps, enhancing patient care, and shaping future research endeavors in this domain.

Treatment of advanced heart failure in adults with congenital heart disease: a narrative review and clinical cases.

Background and Objective: The number of adults with congenital heart disease (ACHD) is increasing worldwide. Almost all congenital cardiac lesions can be successfully treated due to the progress in neonatal surgery and pediatric cardiology with a high likelihood of surviving until adulthood. However, ACHD frequently develop sequelae related to the initial cardiac anomaly. Heart failure (HF) is one of the most common complications associated with a high morbidity and mortality. Methods: The authors did search the PubMed database regarding relevant content covering publications up to March 2022. Relevant manuscripts were classified according to the impact factor of the journal, being a guideline manuscript, a position paper by a society or a comprehensive review of the current literature. Key Content and Findings: Optimal HF treatment remains an unmet need in ACHD. In particular, advanced HF therapy with cardiac resynchronization therapy, ventricular assist devices or organ transplantation is still very different and more specific in ACHD compared to non-ACHD. This review aims to compile international views and evidence from the literatures on the treatment of advanced HF in ACHD. Current challenges, but also the success of different treatment strategies in ACHD are illustrated by clinical cases. Conclusions: The main finding of the review is that data is still scarce regarding ACHD with advanced HF and international efforts to collect data regarding these patients needed to improve the current standard of care.

Myocardial Perfusion in Hypoplastic Left Heart Syndrome.

BACKGROUND: The status of the systemic right ventricular coronary microcirculation in hypoplastic left heart syndrome (HLHS) is largely unknown. It is presumed that the systemic right ventricle's coronary microcirculation exhibits unique pathophysiological characteristics of HLHS in Fontan circulation. The present study sought to quantify myocardial blood flow by cardiac magnetic resonance imaging and evaluate the determinants of microvascular coronary dysfunction and myocardial ischemia in HLHS. METHODS: One hundred nineteen HLHS patients (median age, 4.80 years) and 34 healthy volunteers (median age, 5.50 years) underwent follow-up cardiac magnetic resonance imaging ≈1.8 years after total cavopulmonary connection. Right ventricle volumes and function, myocardial perfusion, diffuse fibrosis, and late gadolinium enhancement were assessed in 4 anatomic HLHS subtypes. Myocardial blood flow (MBF) was quantified at rest and during adenosine-induced hyperemia. Coronary conductance was estimated from MBF at rest and catheter-based measurements of mean aortic pressure (n=99). RESULTS: Hyperemic MBF in the systemic ventricle was lower in HLHS compared with controls (1.89±0.57 versus 2.70±0.84 mL/g per min; P<0.001), while MBF at rest normalized by the rate-pressure product, was similar (1.25±0.36 versus 1.19±0.33; P=0.446). Independent risk factors for a reduced hyperemic MBF were an HLHS subtype with mitral stenosis and aortic atresia (P=0.017), late gadolinium enhancement (P=0.042), right ventricular diastolic dysfunction (P=0.005), and increasing age at total cavopulmonary connection (P=0.022). The coronary conductance correlated negatively with systemic blood oxygen saturation (r, -0.29; P=0.02). The frequency of late gadolinium enhancement increased with age at total cavopulmonary connection (P=0.014). CONCLUSIONS: The coronary microcirculation of the systemic ventricle in young HLHS patients shows significant differences compared with controls. These hypothesis-generating findings on HLHS-specific risk factors for microvascular dysfunction suggest a potential benefit from early relief of frank cyanosis by total cavopulmonary connection.

Heart failure in adults with congenital heart disease: a narrative review.

The number of adults with congenital heart disease (ACHD) has increased over the last decades due to advancements in medical care, including interventional and surgical therapies. We are therefore more frequently challenged by the long-term consequences of palliative or corrective surgery carried out during childhood. Although patients with ACHD may develop conditions related to general cardiovascular risk factors, such as coronary artery disease, the most common complications leading to morbidity and mortality are arrhythmias, heart failure and thromboembolic events. For the management of arrhythmias, current recommendations regarding ablation and device therapy must be considered, whilst also taking into account the anatomical limitations of their congenital heart defect or surgical pathways. Heart failure treatment in acute and chronic settings must also consider the particular anatomy present, including the nature of the systemic ventricle. Treatments strategies for ACHD are typically extrapolated from the respective guidelines in non-ACHD patients, despite a lack of evidence to support this strategy. Right heart failure can be especially challenging to manage in conditions where either a systemic right ventricle or shunt lesions resulting in volume and/or pressure loading of the right ventricle are present. All physicians and cardiologists in particular should be acquainted with the most common diseases in ACHD, their complications and management regime, especially with regards to heart failure as this is a common reason for acute presentation in the emergency department.

Right aortic arch forming a true vascular ring: a clinical review.

OBJECTIVES: This review aims at presenting and summarizing the current state of literature on the presentation and surgical management of a right-sided aortic arch with a left-sided ligamentum forming a complete vascular ring around the oesophagus and trachea. METHODS: A systematic database search for appropriate literature was conducted on PubMed/MEDLINE. Articles were considered relevant when providing details on the presentation, diagnosis and surgical treatment of this specific congenital arch anomaly in human beings. RESULTS: Affected patients present with respiratory and/or oesophageal difficulties due to tracheoesophageal compression. Conservative treatment might be reasonable in asymptomatic or mildly symptomatic cases; however, once moderate-to-severe symptoms develop, surgical intervention is definitely indicated. Surgery is commonly performed through a left thoracotomy or median sternotomy and includes the division of the left ductal ligamentum; if a Kommerell's diverticulum is present that is >1.5 times the diameter of the subclavian artery, then concomitant resection of the large diverticulum and translocation of the aberrant left subclavian artery is also conducted. Postoperative morbidity and mortality are low and are rather related to concomitant intracardiac and extracardiac anomalies than to the procedure itself. In a majority of patients, full resolution of symptoms is seen within months to years from the surgery. Nevertheless, there is also a subset of patients who remain with some tracheobronchial narrowing, sometimes even requiring reintervention during follow-up due to persisting or recurring symptoms. CONCLUSIONS: Overall, the surgical management of a right aortic arch forming a true vascular ring in infancy, childhood and adulthood seems relatively safe and effective in providing symptomatic relief despite some persistent tracheobronchial and/or oesophageal narrowing in some cases.

Narrative review of Ebstein's anomaly beyond childhood: Imaging, surgery, and future perspectives.

Ebstein's anomaly is a rare congenital heart disease with malformation of the tricuspid valve and myopathy of the right ventricle. The septal and inferior leaflets adhere to the endocardium due to failure of delamination. This leads to apical displacement of their hinge points with a shift of the functional tricuspid valve annulus towards the right ventricular outflow tract with a possibly restrictive orifice. Frequently, a coaptation gap yields tricuspid valve regurgitation and over time the "atrialized" portion of the right ventricle may dilate. The highly variable anatomy determines the clinical presentation ranging from asymptomatic to very severe with need for early operation. Echocardiography and magnetic resonance imaging are the most important diagnostic modalities to assess the tricuspid valve as well as ventricular morphology and function. While medical management of asymptomatic patients can be effective for many years, surgical intervention is indicated before development of significant right ventricular dilatation or dysfunction. Onset of symptoms and arrhythmias are further indications for surgery. Modified cone reconstruction of the tricuspid valve is the state-of-the-art approach yielding the best results for most patients. Alternative procedures for select cases include tricuspid valve replacement and bidirectional cavopulmonary shunt depending on patient age and other individual characteristics. Long-term survival after surgery is favorable but rehospitalization and reoperation remain significant issues. Further studies are warranted to identify the optimal surgical strategy and timing before adverse right ventricular remodeling occurs. It is this article's objective to provide a comprehensive review of current literature and an overview on the management of Ebstein's Anomaly. It focuses on imaging, cardiac surgery, and outcome. Additionally, a brief insight into arrhythmias and their management is given. The "future perspectives" summarize open questions and fields of future research.

MRI-based comprehensive analysis of vascular anatomy and hemodynamics.

BACKGROUND: Standardized methods for mapping the complex blood flow in vessels are essential for processing the large data volume acquired from 4D Flow MRI. We present a method for systematic and efficient analysis of anatomy and flow in large human blood vessels. To attain the best outcomes in cardiac surgery, vascular modifications that lead to secondary flow patterns such as vortices should be avoided. In this work, attention was paid to the undesired cancelation of vortices with opposite directions of rotation, known as Dean flow patterns, using hemodynamic parameters such as circulation and helicity density. METHODS: Our approach is based on the multiplanar reconstruction (MPR) of a multi-dimensional feature-space along the blood vessel's centerline. Hemodynamic parameters and anatomic information were determined in-plane from the reconstructed feature-space and from the blood vessel's centerline. A modified calculation of circulation and helicity density and novel parameters for quantifying Dean flow were developed. To test the model performance, we applied our methods to three test cases. RESULTS: Comprehensive information on position, magnitude and interrelation of vascular anatomy and hemodynamics were extracted from 4D Flow MRI datasets. The results show that the Dean flow patterns can be efficiently assessed using the novel parameters. CONCLUSIONS: Our approach to comprehensively and simultaneously quantify multiple parameters of vascular anatomy and hemodynamics from 4D Flow MRI provides new insights to map complex hemodynamic conditions.

Improved Lung Perfusion After Left Pulmonary Artery Patch Enlargement During the Norwood Operation.

BACKGROUND: Optimal pulmonary perfusion is crucial for a well-functioning Fontan circulation in patients with hypoplastic left heart syndrome (HLHS). To obtain an adequate size of the left pulmonary artery (LPA), patch enlargement is a routine part of the hemi-Fontan procedure in our center. However, LPA patch enlargement at the time of the modified Norwood procedure may have surgical advantages. Therefore, the aim of this study was to evaluate whether anatomic and functional effects of the new approach are superior. METHODS: A total of 51 consecutive HLHS patients underwent a cardiovascular magnetic resonance imaging study including assessment of LPA anatomy and lung perfusion. The LPA of 20 patients was enlarged during the modified Norwood procedure (group N) and of 31 patients during the hemi-Fontan procedure (group HF). RESULTS: The median indexed cross-sectional area of the LPA in group N was significantly higher than in group HF (49.5 versus 27.9 mm2/m2, p < 0.0001). The regional pulmonary perfusion as measured by first-pass, contrast-enhanced signal intensity upslope was significantly improved in group N (left side 0.67 s-1 versus 0.40 s-1, p = 0.002; right side 0.84 s-1 versus 0.52 s-1, p = 0.01). The total hemi-Fontan bypass and procedure times were significantly shorter in group N (both p < 0.001). CONCLUSIONS: These first magnetic resonance imaging data show that HLHS patients after LPA patch enlargement during the modified Norwood procedure have significantly higher LPA cross-sectional areas and show improved lung perfusion and shorter overall procedure time as compared with LPA patching during second stage (hemi-Fontan). Therefore, this promising surgical technique may improve blood flow dynamics of the Fontan circulation in the long run.

Aortic valve repair in adult congenital heart disease.

Aortic valve repair in adult congenital heart disease (ACHD) went through a major development during the last two decades to become an increasingly established treatment option in experienced heart valve repair centers. This mini-review addresses valve-sparing treatment strategies in the two most common clinical entities of patients with adult congenital aortic valve disease, namely those presenting with bicuspid (BAV) and unicuspid (UAV) aortic valve disease. Both diseases are integral components of the continuum of congenital aortic valve diseases and represent one of the most common reasons of cardiovascular morbidity in young and otherwise healthy adult patients. The review will highlight the most important advantages of aortic valve sparing procedures as compared to the conventional valve replacement strategy. New treatment aspects will be reviewed including minimally-invasive surgical approaches for aortic valve repair as well as modern protocols of enhanced perioperative recovery which will potentially improve the perioperative recovery and quality of life of the patients undergoing valve-sparing surgical procedures in the future.

"Tailored" endovascular pulmonary valve and root replacement for rupture of a dilated homograft.

Current balloon expandable and self-expanding valves have limitations for the treatment of the enlarged right ventricular outflow tract. We report the first use of a tailored Zenith graft in composition with an Edwards Sapien S3 valve as an alternative to high-risk surgery for the treatment of a spontaneously ruptured homograft in an adult congenital heart disease patient.

A Quarter-Century After Primary Direct Arterial Switch Operation: Four-D-Flow MRI Video Imaging of Blood Flow Dynamics Outcomes.

Four-dimensional (4-D) flow magnetic resonance imaging (MRI) examination was performed 25 years after a neonatal direct arterial switch operation for simple transposition of the great arteries. The 4-D flow MRI video shows physiological spiral anatomical configuration and laminar streamlines in the great arteries.

Restrictive enlargement of the pulmonary annulus at repair of tetralogy of Fallot: a comparative 10-year follow-up study.

OBJECTIVES: Since 1996, our centre performs restrictive enlargement of the pulmonary annulus at surgical repair of tetralogy of Fallot. A transannular patch is only used if the z-score of the pulmonary annulus is smaller than -2. We sought to determine whether this strategy reduces pulmonary insufficiency (PI) and reoperation rate compared to a nationwide contemporary cohort that has not been operated using a uniform strategy. METHODS: Eighty-seven tetralogy of Fallot patients were included in the study (Group 1). One hundred sixty-seven tetralogy of Fallot patients from the Competence Network for Congenital Heart Disease served as controls (Group 2). Clinical, echocardiographic, electrocardiogram, cardiovascular magnetic resonance and outcome data were analysed. RESULTS: Follow-up time since repair was not different between groups [12.9 (7.8-18.8) vs 13.1 (5.2-16.9) years, P = 0.96] while transannular patch rate was significantly lower in Group 1 (32.2% vs 64.7%, P < 0.001). Ten-year freedom from reoperation for PI was significantly higher in our cohort (98% vs 92%, P = 0.01). Multivariable analysis identified restrictive enlargement as the only predictor for no need of reoperation [hazard ratio 0.4 (95% confidence interval 0.24-0.84), P < 0.01]. In Group 1, cardiovascular magnetic resonance-derived volumes were smaller (indexed end-diastolic volume: 103 ± 24 vs 123 ± 31 ml/m2, P < 0.001; indexed end-systolic volume: 53 ± 19 vs 59 ± 20 ml/m2, P = 0.04) and regurgitation fraction was lower (21 ± 14 vs 31 ± 17%, P < 0.001). CONCLUSIONS: Restrictive enlargement of the pulmonary annulus reduces PI and limits the amount of right ventricular dilatation at intermediate-term follow-up. The need for reoperation to alleviate PI is significantly lower compared to a contemporary cohort. Restrictive enlargement strategy has a stronger influence on freedom from reoperation than the use of a transannular patch. CLINICAL TRIAL REGISTRATION: WHO Main ID: DRKS00010087. URL: http://apps.who.int/trialsearch/Trial2.aspx?TrialID=DRKS00010087.

Pericarditis in a Child with an Intrapericardial Bronchogenic Cyst.

Background An intrapericardial bronchogenic cyst (IBC) is a rare congenital malformation seemingly asymptomatic in most subjects. Case Description A 9-year-old boy presented with persistent chest pain and fever. Imaging revealed a large pericardial effusion with a tumor located at the left atrial appendage extending behind the great vessels. Mass rupture with scattered mucoid debris was found intra-operatively. The tumor was excised and the IBC was confirmed by immunohistochemistry. A postcardiotomy syndrome occurred four weeks post-operative. It was treated conservatively. Since then, follow-up visits have remained uneventful. Conclusion IBCs are a scarce, but serious cause of persistent inflammation in children.

Is the Lecompte technique the last word on transposition of the great arteries repair for all patients? A magnetic resonance imaging study including a spiral technique two decades postoperatively.

OBJECTIVES: To compare the Lecompte technique and the spiral anastomosis (complete anatomic correction) two decades after arterial switch operation (ASO). METHODS: Nine patients after primary ASO with Lecompte and 6 selected patients after spiral anastomosis were evaluated 20.8 ± 2.1 years after ASO versus matched controls. Blood flow dynamics and flow profiles (e.g. vorticity, helicity) in the great arteries were quantified from time-resolved 3D magnetic resonance imaging (MRI) phase contrast flow measurements (4D flow MR) in addition to a comprehensive anatomical and functional cardiovascular MRI analysis. RESULTS: Compared with spiral reconstruction, patients with Lecompte showed more vortex formation, supranatural helical blood flow (relative helicity in aorta: 0.036 vs 0.089; P < 0.01), a reduced indexed cross-sectional area of the left pulmonary artery (155 vs 85 mm²/m²; P < 0.001) and more semilunar valve dysfunctions (n = 5 vs 1). There was no difference in elastic aortic wall properties, ventricular function, myocardial perfusion and myocardial fibrosis between the two groups. Cross-sectional area of the aortic sinus was larger in patients than in controls (669 vs 411 mm²/m²; P < 0.01). In the spiral group, the pulmonary root was rotated after ASO more towards the normal left position (P < 0.01). CONCLUSIONS: In this study, selected patients with spiral anastomoses showed, two decades after ASO, better physiologically adapted blood flow dynamics, and attained a closer to normal anatomical position of their great arteries, as well as less valve dysfunction. Considering the limitations related to the small number of patients and the novel MRI imaging techniques, these data may provoke reconsidering the optimal surgical approaches to transposition of the great arteries repair.

Frequent Dilatation of the Descending Aorta in Children With Hypoplastic Left Heart Syndrome Relates to Decreased Aortic Arch Elasticity.

BACKGROUND: Patients with hypoplastic left heart syndrome after a Norwood operation show dilatation and reduced distensibility of the reconstructed proximal aorta. Cardiac magnetic resonance imaging (CMR) and angiographic examinations indicate that the native descending aorta (DAo) is also dilated, but this has not been studied in detail. METHODS AND RESULTS: Seventy-nine children with hypoplastic left heart syndrome in Fontan circulation (aged 6.3±3.2 years) and 18 control participants (aged 6.8±2.4 years) underwent 3.0-tesla CMR. Gradient-echo cine and phase-contrast imaging was applied to measure cross-sectional areas (CSAs), distensibility, pulse wave velocity, and the incremental elastic modulus of the thoracic aorta. CSA of the DAo in patients was also compared with published percentiles for aortic CSA. Patients had significantly larger CSA of the DAo at the level of pulmonary artery bifurcation (229.1±97.2 versus 175.7±24.3 mm/m(2), P=0.04) and the diaphragm (196.2±66.0 versus 142.6±16.7 mm/m(2), P<0.01). In 41 patients (52%), CSA of the DAo was >95th percentile level for control participants, and the incremental elastic modulus of the aortic arch and the DAo was higher than in patients with normal CSAs (arch: 90.1±64.3 versus 45.6±38.9 m/s; DAo: 86.3±53.7 versus 47.1±47.6 m/s; P<0.01). Incremental elastic modulus of the aortic arch and the DAo correlated with the CSA of the DAo (arch: r=0.5; DAo: r=0.49; P<0.01). CONCLUSIONS: Children with hypoplastic left heart syndrome frequently show dilatation of their DAo associated with increased stiffness of the aortic arch. Higher aortic impedance increases the afterload of the systemic circulation and likely contributes to the burden of the systemic right ventricle.

Arterial elastance and its impact on intrinsic right ventricular function in palliated hypoplastic left heart syndrome.

BACKGROUND: Systemic right ventricular (RV) function is a major determinant of long-term outcome in patients with palliated hypoplastic left heart syndrome (HLHS). Abnormal elastic properties of the reconstructed aorta may negatively impact on ventricular function. We therefore aimed to assess arterial elastance and its relationship to systemic RV function in these patients. METHODS: Fifty-six HLHS patients (median age 5.4 years; range 2.9-14.2 years) were studied at a median of 2.6 years (range 0.8-12.7 years) after completion of the Fontan circulation with the pressure-volume conductance system. RESULTS: Arterial elastance (Ea) was abnormally high and correlated inversely with RV ejection fraction (r=-0.42, P=0.001). However, end systolic elastance (Ees) - a load independent measure of intrinsic systolic ventricular function - and more so end diastolic stiffness (Eed) were positively correlated with Ea (Ees vs. Ea: r=0.44, P=0.001: Eed vs. Ea: r=0.62, P<0.0001). Patients who were treated for significant aortic arch obstruction after surgical palliation showed higher Ea and Eed even four years after successful treatment compared to the remainder of the group (Ea: 3.4 ± 1.2 vs. 2.8 ± 1.0 mmHg/ml, P=0.04 and Eed: 0.67 ± 0.44 vs. 0.45 ± 0.3 mmHg/ml, P=0.04). CONCLUSIONS: Arterial elastance is abnormally high in palliated HLHS patients and negatively impacts on ejection fraction but not on intrinsic systolic RV function early after completion of the Fontan circulation. Increased arterial elastance, however, is associated with increased RV diastolic stiffness with potential adverse effects on long-term outcome. Furthermore, arterial elastance and diastolic stiffness are particularly high in patients who needed treatment for aortic arch obstruction.

Adverse results of a decellularized tissue-engineered pulmonary valve in humans assessed with magnetic resonance imaging.

OBJECTIVES: Matrix P® and Matrix P plus® tissue-engineered pulmonary valves (TEPV) were offered as an improvement for pulmonary valve replacement (PVR) because of recellularization by host cells. The high frequency of graft failure gave reason to evaluate the underlying morphological substrate using magnetic resonance imaging (MRI) and histology. METHODS: Between June 2006 and August 2008, 17 Matrix P® and 10 Matrix P plus® TEPVs were implanted in 26 patients with a median age of 12.4 (range: 0.8-38.7, interquartile range: 6.1-18.1) years. The grafts were studied by MRI, and underwent histological examination when explantation was required. RESULTS: Surgical (n = 13) or transcatheter (n = 1) TEPV replacement because of graft failure was needed in 14 cases (52%) 19 (0.5-53) months after implantation. MRI detected significant TEPV stenosis with mild insufficiency (V(max) = 3.7 ± (standard deviation) 0.5 m/s, regurgitant fraction (RGF) = 10 ± 3%) and stenosis with moderate-to-severe insufficiency (V(max) = 3.5 ± 0.8 m/s, RGF = 38 ± 10%) in 6 patients, respectively, and severe insufficiency (RGF = 40%) in 1 patient. In patients with graft failure, MRI showed hyperenhancement and TEPV wall thickening. Histology revealed severe inflammation, increased fibrous tissue and foreign-body reaction against valve leaflets and fascial tissue, while TEPV endothelialization was not detected in any case. CONCLUSIONS: The high frequency of Matrix P® and Matrix P plus® graft failure can be related to inflammation and fibrosis revealed by MRI and histology. Our results do not support the use of these valves for PVR and suggest careful follow-up examinations, including MRI for early detection of graft inflammation and fibrosis.

Anatomical and functional assessment of the intra-atrial lateral tunnel in the Fontan circulation.

OBJECTIVES: In patients after completion of the total cavopulmonary connection (TCPC) with an intra-atrial lateral tunnel, deviations of the tunnel from an ideal straight tubular shape were observed. However, little is known about frequency and adverse effects of such shape deviations. We sought to analyse tunnel anatomy, dimensions and blood flow using cardiac magnetic resonance imaging (CMR). METHODS: Fifty-four patients with hypoplastic left heart syndrome (HLHS; mean age 6.0 ± 2.4 years) underwent CMR with gradient-echo cine sequences, 2D- and 3D-phase-contrast imaging. We analysed anatomy, diameters, cross-sectional areas, volumes and blood flow of the tunnel. RESULTS: Twenty-five patients had a tubular tunnel. In 29 patients, bulging and/or narrowing of the tunnel were present. Cross-sectional areas and volumes of the tunnel were not significantly different between the two groups. There were also no differences for the mean blood flow and the mean and maximal flow velocity (P = 0.05-0.6). In all the patients, the normalized tunnel volume was related to age (r = 0.44; P = 0.002) and body surface area (BSA; r = 0.42; P = 0.005). The mean tunnel blood flow correlated with age (r = 0.73; P = 0.001) and BSA (r = 0.83; P < 0.0001). CONCLUSIONS: A considerable percentage of patients with an intra-atrial lateral tunnel develop mild deviations of the tunnel from ideal tubular shape. The correlation between tunnel volume and mean blood flow with age and BSA suggests that the capacity of the tunnel adjusts to body growth, independent of tunnel shapes that deviate from a fluid-dynamically favourable shape. Follow-up CMRs are needed to detect long-term effects of irregular tunnel shapes on flow dynamics.

Does left ventricular size impact on intrinsic right ventricular function in hypoplastic left heart syndrome?

BACKGROUND: The size of the remnant left ventricle (LV) may influence right ventricular function and thus long-term outcome in palliated hypoplastic left heart syndrome (HLHS). We therefore sought to assess the impact of the size of the hypoplastic LV on intrinsic RV function in HLHS patients after Fontan surgery. METHODS: Fifty-seven HLHS patients were studied 2.5 (range: 0.8-12.6) years after Fontan-type palliation with the pressure-volume conductance system. The patient cohort was divided into two groups according to the median LV area index (group 1: LV area index ≤ 1.33 cm(2)/m(2), n=29; group 2: LV area index>1.33 cm(2)/m(2), n=28). RESULTS: The slopes of the end systolic elastance (Ees) and the preload recruitable stroke work relation (Mw) were not different between group 1 and 2 (Ees: 2.70 ± 1.92 vs. 3.68 ± 2.68 mmHg/ml; Mw: 52.75 ± 14.98 vs. 51.09 ± 16.63 mmHg x ml; P=NS for all). Furthermore, the systolic responses to dobutamine were not statistically different between groups. However, the slope of the end diastolic stiffness (Eed) was higher in group 2 and catecholaminergic stimulation resulted in a decrease in Eed in group 2 (group 1: 0.40 ± 0.26 vs. 0.52 ± 0.45; group 2: 0.68 ± 0.44 vs. 0.47 ± 0.38 mmHg/ml, P<0.01). Furthermore Eed was lowest in patients with mitral atresia/aortic atresia, the anatomic subgroup with the smallest LV remnant. CONCLUSIONS: Intrinsic systolic RV function is not affected by the size of the hypoplastic LV in survivors of surgical palliation of HLHS. Diastolic stiffness, however, was higher in patients with a larger LV remnant.